Elsberg Syndrome with Mixed Presentation as Meningitis Retention Syndrome: A Pediatric Case Report and Comprehensive Review of the Literature

Abstract

Elsberg syndrome is a typically infectious syndrome that may cause acute or subacute bilateral lumbosacral radiculitis and sometimes lower spinal cord myelitis. Patients often present with various neurological symptoms involving the lower extremities, including numbness, weakness, and urinary disturbances such as retention. A 9-year-old girl with no significant past medical history presented with altered mental status, fever, urinary retention, and anuria and was found to have encephalomyelitis. An extensive diagnostic workup led to ruling out possible etiologies until identifying Elsberg syndrome. In this report, we describe a case of Elsberg syndrome caused by West Nile virus (WNV). To the best of our knowledge, this is the first reported case of its kind in the pediatric population. Utilizing PubMed and Web of Science databases, we reviewed the literature to describe the neurogenic control of the urinary system in correlation to a multitude of neurologic pathologies.

Keywords: Elsberg syndrome; West Nile virus; encephalopathy; meningitis retention syndrome (MRS); meningoencephalitis; myeloencephalitis; urinary neuroanatomy; urinary retention.

Figure 1

Initial axial DWI/ADC maps demonstrate subtle small area of restricted diffusion in left posterior temporal lobe (white arrows in images A,B) and without perceivable enhancement (image C). There is additional subtle T2 prolongation in the dorsal pons in the midline and paramedian region (white arrow image D). Initial sagittal STIR image of the thoracic spine demonstrates scattered patchy areas of T2 prolongation with worst involvement of the lower cord and conus medullaris (white arrows in images E,F). Additional patchy T2 prolongation is seen in left lateral cervical spinal cord at C5 (white arrow in image G).

Figure 2

Repeat neuroaxis imaging shows increasing patchy restricted diffusion in left posterior temporal and occipital lobes (white arrows in images A,B). Small area in left posterior temporal lobe which demonstrated restricted diffusion on prior MRI brain shows increasing T2 prolongation (white arrow in image C). There is no perceivable enhancement (image D). Repeat imaging showing dorsal medulla in the expected location of area postrema (white arrow in image E). Axial T2 image through the lower cervical spinal cord demonstrates cord swelling and central cord T2 prolongation (black arrow in image F). Sagittal STIR image of the cervical spine shows multifocal areas of T2 prolongation with cord swelling (white arrow in image G) and is also noted in extensive T2 prolongation in the dorsal brain stem (red arrow image G). Axial T2 image through the conus medullaris demonstrates T2 prolongation with cord swelling (white arrow in image H) but without perceivable enhancement (white arrow in image I).

Figure 3

Brain structures involved with micturition.

Figure 4

Activity and control involved during micturition.